Stock
FESA Number
1974
Strain Name
129.C-Tg(Prnp/PRNP)1Drb 129-Prnp<tm1Cwe>/DrbH
Mutation Strain Type
Attribution
Gene/Allele Information
| Allele Name | Allele MGI ID | Gene Name | Gene MGI ID | Chromosome |
|---|---|---|---|---|
| Prnp<tm1Cwe> | MGI:1888773 | Prnp | MGI:97769 | 2 |
Phenotypic Description
The octameric repeat region of the mouse prion protein was replaced with the hexameric repeat region from the domestic chicken. The mice express mouse PrP at wild-type levels with correct membrane location and orientation but have an altered metal binding region. The mice have been generated to get a better understanding of prion diseases such as CJD in humans.
Orphanet Disorders
| OrphaNet ID | Name | Url |
|---|---|---|
| 356 | Gerstmann-Straussler-Scheinker syndrome | http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=356 |
| 466 | Fatal familial insomnia | http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=466 |
| 157 941 | Huntington disease-like 1 | http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=157941 |
| 280 397 | Familial Alzheimer-like prion disease | http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=280397 |
| 282 166 | Inherited Creutzfeldt-Jakob disease | http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=282166 |
Publications
Displaying 201 - 210 of 226 Stock Publications
| Pubmed ID | Authors | Title | Journal |
|---|---|---|---|
| 12758067 | Curtis J | Age-dependent loss of PTP and LTP in the hippocampus of PrP-null mice. | Neurobiol Dis (2 003) 13:55-62 |
| 22072968 | Friedman-Levi Y | Fatal prion disease in a mouse model of genetic E200K Creutzfeldt-Jakob disease. | PLoS Pathog (2 011) 7:e1002350 |
| 10987828 | Herms JW | Altered intracellular calcium homeostasis in cerebellar granule cells of prion protein-deficient mice. | J Neurochem (2 000) 75:1487-92 |
| 16135751 | Kong Q | Chronic wasting disease of elk: transmissibility to humans examined by transgenic mouse models. | J Neurosci (2 005) 25:7944-9 |
| 9754876 | Lipp HP | A 2-year longitudinal study of swimming navigation in mice devoid of the prion protein: no evidence for neurological anomalies or spatial learning impairments. | Behav Brain Res (1 998) 95:47-54 |
| 15951190 | Mayer-Sonnenfeld T | The metabolism of glycosaminoglycans is impaired in prion diseases. | Neurobiol Dis (2 005) 20:738-43 |
| 15351724 | Nishimura T | Cellular prion protein regulates intracellular hydrogen peroxide level and prevents copper-induced apoptosis. | Biochem Biophys Res Commun (2 004) 323:218-22 |
| 21277354 | Ratté S | Threshold for epileptiform activity is elevated in prion knockout mice. | Neuroscience (2 011) 179:56-61 |
| 19073920 | Sigurdson CJ | De novo generation of a transmissible spongiform encephalopathy by mouse transgenesis. | Proc Natl Acad Sci U S A (2 009) 106:304-9 |
| 14747574 | Tremblay P | Mutant PrPSc conformers induced by a synthetic peptide and several prion strains. | J Virol (2 004) 78:2088-99 |
Pages
Orphanet Categories
Rare neurologic disease
EMMA ID
EM:02421
Genetic Status
GMO
Background Strain Name
129
Background Strain MGI ID
MGI:2160041
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